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Jervell and Lange-Nielsen Syndrome

National Organization for Rare Disorders, Inc.

ImportantIt is possible that the main title of the report Jervell and Lange-Nielsen Syndrome is not the name you expected.

Disorder Subdivisions

None

General Discussion

Summary

Jervell and Lange-Nielsen syndrome (JLNS) is a rare inherited disorder characterized by deafness present at birth (congenital) occurring in association with abnormalities affecting the electrical system of the heart. The severity of cardiac symptoms associated with JLNS varies from case to case. Some individuals may have no apparent symptoms (asymptomatic); others may develop abnormally increased heartbeats (tachyarrhythmias) resulting in episodes of unconsciousness (syncope), cardiac arrest, and potentially sudden death. Physical activity, excitement, fright, or stress may trigger the onset of these symptoms. Fainting during the aforementioned activities is also a classic sign of JLNS. JLNS is usually detected during early childhood and is inherited as an autosomal recessive genetic disorder. More than half of the untreated cases of JLNS result in death before the age of 15.

Introduction

Anton Jervell and Fred Lange-Nielsen provided the first complete description of the electrical conduction problem in the heart called long QT syndrome (LQTS) in 1957. LQTS refers to the QT-interval measured on the electrocardiogram that indicates that the heart muscle is taking longer than usual to recharge between beats. It predisposes those affected to tachyarrhythmias called torsade de pointes (TdP) which leads to syncope and may cause sudden cardiac death.

Supporting Organizations

American Academy of Audiology

11480 Commerce Park Drive

Suite 220

Reston, VA 20191

Tel: 703-790-8466

Fax: 703-790-8631

Tel: 800-222-2336

Email: infoaud@audiology.org

Website: http://www.audiology.org

American Heart Association

7272 Greenville Avenue

Dallas, TX 75231

Tel: (214)784-7212

Fax: (214)784-1307

Tel: (800)242-8721

Email: Review.personal.info@heart.org

Website: http://www.heart.org

Fetal Hope Foundation

9786 South Holland Street

Littleton, CO 80127

USA

Tel: (303)932-0553

Tel: (877)789-4673

Email: info@fetalhope.org

Website: http://www.fetalhope.org

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

Website: http://rarediseases.info.nih.gov/GARD/

Hearing Health Foundation

363 Seventh Avenue, 10th Floor

New York, NY 10016-3904

United States

Tel: (212)257-6140

Fax: (212)257-6139

Tel: (866)454-3924

Email: info@hearinghealthfoundation.org

Website: http://hearinghealthfoundation.org/home

International Long QT Syndrome Registry

Heart Research Follow-up Program

Box 653

Rochester, NY 14642-8653

Tel: (585)276-0016

Fax: (585)273-5283

Email: heartajm@heart.rochester.edu

NIH/National Heart, Lung and Blood Institute

P.O. Box 30105

Bethesda, MD 20892-0105

Tel: (301)592-8573

Fax: (301)251-1223

Email: nhlbiinfo@rover.nhlbi.nih.gov

Website: http://www.nhlbi.nih.gov/

QTsyndrome.ch

Ronnerweg 2

Nidau, 2560

Switzerland

Tel: 41794741535

Email: info@qtsyndrome.ch

Website: http://www.qtsyndrome.ch/index.html

Sudden Arrhythmia Death Syndromes Foundation

508 E. South Temple

Suite 202

Salt Lake City, UT 84102

USA

Tel: (801)531-0937

Fax: (801)531-0945

Tel: (800)786-7723

Email: laura@sads.org

Website: http://www.sads.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

Last Updated: 1/16/2013Copyright 2013 National Organization for Rare Disorders, Inc.

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